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Outcomes of Bypass Surgery in Patients with Moyamoya Syndrome Secondary To Sickle Cell Disease: a Multicenter Study
Basel Musmar1, Joanna M Roy1, Hammam Abdalrazeq2
1Department of Neurological Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA.
Background:
Moyamoya syndrome (MMS) associated with sickle cell disease (SCD) is a severe vasculopathy that significantly increases stroke risk. While cerebral revascularization is increasingly considered in this population, concerns about perioperative safety and long-term outcomes have limited its use in clinical practice.
Methods:
We conducted a multicenter, retrospective cohort study of 553 patients with MMS who underwent surgical revascularization across 13 centers. Patients were grouped by SCD status (SCD-MMS vs. moyamoya disease (MMD)). Primary outcomes included perioperative stroke, perioperative complications, and functional status at discharge. Secondary outcomes included length of stay, and follow-up stroke.
Results:
Of 553 patients, 32 (5.8%) had SCD. There were no significant differences in overall perioperative stroke (OR 1.05, 95% CI 0.19 to 5.54), symptomatic perioperative stroke (OR 0.94, 95% CI 0.09 to 8.94), perioperative complications (OR 1.66, 95% CI 0.47 to 5.86), or follow-up stroke (OR 0.88, 95% CI 0.17 to 4.55). Functional outcomes at discharge were similarly favorable in both groups (mRS 0-1: OR 0.84, 95% CI 0.29 to 2.40). SCD was associated with a longer hospital stay (beta 2.78 days, 95% CI 0.60 to 4.96).
Conclusion:
Surgical revascularization for MMS in patients with SCD does not confer additional procedural risk and yields outcomes comparable to those of patients without SCD. These findings support the role of bypass surgery as a viable treatment option in this high-risk population.
Insights
Surgical revascularization for moyamoya syndrome (MMS) in sickle cell disease (SCD) patients is safe and effective. Outcomes are comparable to non-SCD patients, supporting bypass surgery for this high-risk group.
Area of Science:
- Neurology
- Vascular Surgery
- Hematology
Background:
- Moyamoya syndrome (MMS) with sickle cell disease (SCD) presents a severe vasculopathy with high stroke risk.
- Cerebral revascularization is considered, but perioperative safety and long-term outcomes are concerns in SCD patients.
Purpose of the Study:
- To evaluate the safety and efficacy of surgical revascularization in patients with MMS associated with SCD.
- To compare perioperative and long-term outcomes between SCD-MMS and moyamoya disease (MMD) patients.
Main Methods:
- A multicenter, retrospective cohort study of 553 MMS patients undergoing surgical revascularization.
- Patients were stratified by SCD status (SCD-MMS vs. MMD).
- Primary outcomes included perioperative stroke, complications, and functional status; secondary outcomes included length of stay and follow-up stroke.
Main Results:
- No significant differences in perioperative stroke, symptomatic stroke, or complications between SCD-MMS and MMD groups.
- Functional outcomes at discharge were comparable (mRS 0-1).
- SCD-MMS patients experienced a longer hospital stay.
Conclusions:
- Surgical revascularization for MMS in SCD patients does not increase procedural risk.
- Outcomes are comparable to non-SCD patients, supporting bypass surgery as a viable treatment.
- This approach is a beneficial option for this high-risk population.
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